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Cori disease is the type III of glycogen storage disease (GSD)

Overview:
Cori disease is the type III of glycogen storage disease (GSD) which results from deficiency of the enzyme
that convert glycogen to glucose which is called debranching enzyme. That leads to glycogen accumulation in tissues.
In many cases, this deficiency has systemic consequences, but, in some cases, the defect is limited to specific tissues.

The other common type of glycogen storage diseases is Von Gierke disease.


Symptoms:
The most common symptoms are muscle weakness and muscle cramps .
Cori disease cause hypoglycemic seizures and cardiomegaly.
The accumulation of glycogen in body’s cells impairs the function of certain organs and tissues, especially the liver and muscles.
Infants with type of GSDIII may have low blood sugar (hypoglycemia), increase of amounts of fats in the blood (hyperlipidemia), and elevated enzymes levels of liver.
When they get older, the disease develop an enlarged liver (hepatomegaly) which return to its normal size during adolescence, but sometimes some the disease cause  chronic liver disease (cirrhosis) and liver failure later in life.
As a result of liver problems, infected people will have slow growth which can lead to short stature.
In rare cases GSGIII can cause noncancerous (benign) tumors called adenomas may form in the liver.

Causes:
Cori disease is an inherited disease caused by accumulation of  complex sugar called glycogen in the body's cells. And thus deficiency of glucose in blood.

Diagnosis:

Treatment
Regular feeding, including overnight, and management of associated conditions.
For  Treatment of glycogen storage disease type III , we can use high-protein diet, to enhance gluconeogenesis.
Cori disease is the type III of glycogen storage disease (GSD) Reviewed by Tpdsm on September 20, 2017 Rating: 5

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